Rcan1 Deficiency Impairs Neuronal Migration and Causes Periventricular Heterotopia

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	Rcan1 Deficiency Impairs Neuronal Migration and Causes Periventricular Heterotopia
	Li, Yang; Wang, Jie; Zhou, Yang; Li, Dan; Xiong, Zhi-Qi
刊名	JOURNAL OF NEUROSCIENCE
	2015
卷号	35 期号:2 页码:610-620
关键词	SYNDROME CRITICAL REGION NEURAL PROGENITOR PROLIFERATION LONG-TERM POTENTIATION DOWN-SYNDROME NODULAR HETEROTOPIA CORTICAL-NEURONS CEREBRAL-CORTEX CELL MIGRATION FILAMIN-A CALCINEURIN
通讯作者	Xiong, ZQ (reprint author), Chinese Acad Sci, Inst Neurosci, 320 Yueyang Rd, Shanghai 200031, Peoples R China.
英文摘要	Periventricular heterotopia (PH) is a cortical malformation characterized by aggregation of neurons lining the lateral ventricles due to abnormal neuronal migration. The molecular mechanism underlying the pathogenesis of PH is unclear. Here we show that Regulators of calcineurin 1 (Rcan1), a Down syndrome-related gene, plays an important role in radial migration of rat cortical neurons. Downregulation of Rcan1 by expressing shRNA impaired neural progenitor proliferation and led to defects in radial migration and PH. Two isoforms of Rcan1 (Rcan1-1 and Rcan1-4) are expressed in the rat brain. Migration defects due to downregulation of Rcan1 could be prevented by shRNA-resistant expression of Rcan1-1 but not Rcan1-4. Furthermore, we found that Rcan1 knockdown significantly decreased the expression level of Flna, an F-actin cross-linking protein essential for cytoskeleton rearrangement and cell migration, mutation of which causes the most common form of bilateral PH in humans. Finally, overexpression of FLNA in Rcan1 knockdown neurons prevented migration abnormalities. Together, these findings demonstrate that Rcan1 acts upstream from Flna in regulating radial migration and suggest that impairment of Rcan1-Flna pathway may underlie PH pathogenesis.
学科主题	Neurosciences
WOS标题词	Neurosciences & Neurology
收录类别	SCI
语种	英语
WOS记录号	WOS:000349409300017
公开日期	2015-05-27
内容类型	期刊论文
源URL	[http://ir.sibs.ac.cn/handle/331001/2668]
专题	上海神经科学研究所_神经所(总)
推荐引用方式 GB/T 7714	Li, Yang,Wang, Jie,Zhou, Yang,et al. Rcan1 Deficiency Impairs Neuronal Migration and Causes Periventricular Heterotopia[J]. JOURNAL OF NEUROSCIENCE,2015,35(2):610-620.
APA	Li, Yang,Wang, Jie,Zhou, Yang,Li, Dan,&Xiong, Zhi-Qi.(2015).Rcan1 Deficiency Impairs Neuronal Migration and Causes Periventricular Heterotopia.JOURNAL OF NEUROSCIENCE,35(2),610-620.
MLA	Li, Yang,et al."Rcan1 Deficiency Impairs Neuronal Migration and Causes Periventricular Heterotopia".JOURNAL OF NEUROSCIENCE 35.2(2015):610-620.